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Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia

Year 2021, Volume: 5 Issue: 1, 89 - 92, 01.01.2021
https://doi.org/10.28982/josam.865468

Abstract

Background/Aim: Non-classic congenital adrenal hyperplasia (CAH) is a milder form of CAH. The CYP21A2 gene is involved in the etiopathogenesis of both severe (classic) and mild (non-classic) form CAH, however, genetic mutations in non-classic CAH result in less impairment of 21-hydroxylase activity. Therefore, as in classic CAH, patients with non-classical CAH have no signs and symptoms of cortisol deficiency. Instead, there may be signs of hyperandrogenism that can appear later in childhood or in early adulthood. Due to excess androgenic effect on clitoris, labia minora, majora and the vagina, feminizing genitoplasty should be performed to make gender identities consistent and reshape the proper genital anatomy for sexual intercourse. However, there are few studies and controversy on feminizing genitoplasty procedures in adulthood. In this study, we aimed to design a new procedure to spare the nerves of the clitoris as well as the clitoral body, along with the cavernous tissue.
Methods: This is a case series of nine patients with a mean age of 24.8 years diagnosed with non-classic CAH who underwent feminizing genitoplasty, in which nerve-sparing clitoroplasty was performed with corporal septum excision by ventral approach. Initially, diagnostic cystoscopy was performed to detect the level of vaginal confluence into the urogenital sinus. The enlarged clitoris was degloved from 10 mm proximal to the glans up to the symphysis pubis. Corporal septum was excised from the ventral part of the clitoris up to the bifurcation of crura. Neurovascular bundle was preserved completely dorsally, and the clitoris was folded over itself and fixed at the level of crural bifurcation at 3 and 9 o'clock positions. Degloved clitoral preputium was used as Byars’ flaps for labiaplasty. A perineal inverted U incision was made and the vaginal introitus was enlarged with this flap. Female Genital Image Scale (FGIS) was used in the assessment of patients’ postoperative genital self-image.
Results: Feminizing genitoplasty (nerve-sparing clitoroplasty with corporal septum excision, labiaplasty and perineal flap vaginoplasty) was performed in nine patients diagnosed with non-classic CAH. The mean operation time was 112 minutes with a range of 90-140 minutes. Urogenital sinus mobilization was not performed as the vaginal confluence into urogenital sinus was low in cystoscopy. Patients were re-assessed at 1 month, 3 months and 6 months postoperatively. FGIS scores showed that four patients were “very satisfied,” one patient was “satisfied,” 2 patients were “moderately satisfied”, and one patient was “dissatisfied.” The maximum follow up was 2 years with no recorded short or long-term complications.
Conclusion: Nerve sparing clitoroplasty with corporal septum excision is a good option with satisfactory long term results for non-classic CAH patients. However, we need many more comparative studies to decide the gold standard method for optimal physiologic and cosmetic outcomes in CAH patients.

References

  • 1. Eckoldt-Wolke F. Timing of surgery for feminizing genitoplasty in patients suffering from congenital adrenal hyperplasia. Endocr Dev. 2014;27:203-9. doi: 10.1159/000363664. Epub 2014 Sep 9. PMID: 25247657
  • 2. Therrell BL. Newborn screening for congenital adrenal hyperplasia. Endocrinol Metab Clin North Am.2001;30:15. doi: 10.1016/S0889-8529(08)70017-3.
  • 3. Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet. 2005;365:2125. doi: 10.1016/S0140-6736(05)66736-0.
  • 4. Jaaskelainen J, Levo A, Voutilainen R, Partanen J. Population-wide evaluation of disease manifestation in relation to molecular genotype in steroid 21-hydroxylase (CYP21) deficiency: good correlation in a well defined population. J Clin Endocrinol Metab. 1997;82:3293.
  • 5. Morrison TG, Bearden A, Ellis SR, Harriman R. Correlates of genital perceptions among Canadian post-secondary students. Electronic Journal of Human Sexuality. 2005;8. Retrieved June 2, 2008, from http://www.ejhs.org/volume8/GenitalPerceptions.htm.
  • 6. Brentnall CP. Case of arrhenoblastoma complicating pregnancy. J Obstet Gynaecol Br Emp. 1945;52:235.
  • 7. Persechini ML, Motton S, Leguevaque P, Donadille F, Escourrou G, Vierasu B, et al. Virilising ovarian tumour: a case associating a Sertoli-Leydig cell tumour and a Brenner tumour. Gynecol Endocrinol. 2011;27:345.
  • 8. Kunz GJ, Klein KO, Clemons RD, Gottschalk ME, Jones KL. Virilization of young children after topical androgen use by their parents. Pediatrics. 2004;114:282.
  • 9. Kearse WS Jr, Ritchey ML. Clitoral enlargement secondary to neurofibromatosis. Clin Pediatr (Phila). 1993;32:303.
  • 10. Williams CE, Nakhal RS, Achermann JC, Creighton SM. Persistent unexplained congenital clitoromegaly in females born extremely prematurely. J Pediatr Urol. 2013;9:962.
  • 11. Phillip M, De Boer C, Pilpel D, Karplus M, Sofer S. Clitoral and penile sizes of full term newborns in two different ethnic groups. J Pediatr Endocrinol Metab. 1996 Mar-Apr;9(2):175-9.
  • 12. Kutlu HA, Akbiyik F. Clitoral length in female newborns: A new approach to the assessment of clitoromegaly. Turk J Med Sci. 2011;41:495-9.
  • 13. Dickinson RL.Atlas of Human Sex Anatomy. 2nd Ed. Baltimore: Williams & Wilkins. 1949; p. 1–145.
  • 14. Lloyd J, Crouch NS, Minto CL, Liao LM, Creighton SM. Female genital appearance: ‘Normality’ unfolds. BJOG. 2005;112:643–6.
  • 15. Oberfi eld SE, Mondok A, Shahrivar F, Klein JF, Levine LS. Clitoral size in full-term infants. American Journal of Perinatology. 1989;6:453-5.
  • 16. Verkauf BS,Von Thron J,O'Brien WF.Clitoral size in normal women. Obstet Gynecol. 1992;80:41–4.
  • 17. Poppas DP, Hochsztein AA, Baergen RN, Loyd E, Chen J, Felsen D. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol. 2007 Oct;178(4 Pt 2):1802-6; discussion 1806.
  • 18. Creighton SM. Surgery for intersex. J R Soc Med.2001;94:218-20.
  • 19. Crouch NS, Liao LM, Woodhouse CR, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol. 2008;179:634.
  • 20. Crouch NS, Minto CL, Laio LM, Woodhouse CR, Creighton SM. Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study. BJU Int. 2004;93:135.
  • 21. Baskin LS, Erol A, Li YW, Liu WH, Kurzrock E, Cunha GR. Anatomical studies of the human clitoris. J Urol. 1999 Sep;162(3 Pt 2):1015-20.
Year 2021, Volume: 5 Issue: 1, 89 - 92, 01.01.2021
https://doi.org/10.28982/josam.865468

Abstract

References

  • 1. Eckoldt-Wolke F. Timing of surgery for feminizing genitoplasty in patients suffering from congenital adrenal hyperplasia. Endocr Dev. 2014;27:203-9. doi: 10.1159/000363664. Epub 2014 Sep 9. PMID: 25247657
  • 2. Therrell BL. Newborn screening for congenital adrenal hyperplasia. Endocrinol Metab Clin North Am.2001;30:15. doi: 10.1016/S0889-8529(08)70017-3.
  • 3. Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet. 2005;365:2125. doi: 10.1016/S0140-6736(05)66736-0.
  • 4. Jaaskelainen J, Levo A, Voutilainen R, Partanen J. Population-wide evaluation of disease manifestation in relation to molecular genotype in steroid 21-hydroxylase (CYP21) deficiency: good correlation in a well defined population. J Clin Endocrinol Metab. 1997;82:3293.
  • 5. Morrison TG, Bearden A, Ellis SR, Harriman R. Correlates of genital perceptions among Canadian post-secondary students. Electronic Journal of Human Sexuality. 2005;8. Retrieved June 2, 2008, from http://www.ejhs.org/volume8/GenitalPerceptions.htm.
  • 6. Brentnall CP. Case of arrhenoblastoma complicating pregnancy. J Obstet Gynaecol Br Emp. 1945;52:235.
  • 7. Persechini ML, Motton S, Leguevaque P, Donadille F, Escourrou G, Vierasu B, et al. Virilising ovarian tumour: a case associating a Sertoli-Leydig cell tumour and a Brenner tumour. Gynecol Endocrinol. 2011;27:345.
  • 8. Kunz GJ, Klein KO, Clemons RD, Gottschalk ME, Jones KL. Virilization of young children after topical androgen use by their parents. Pediatrics. 2004;114:282.
  • 9. Kearse WS Jr, Ritchey ML. Clitoral enlargement secondary to neurofibromatosis. Clin Pediatr (Phila). 1993;32:303.
  • 10. Williams CE, Nakhal RS, Achermann JC, Creighton SM. Persistent unexplained congenital clitoromegaly in females born extremely prematurely. J Pediatr Urol. 2013;9:962.
  • 11. Phillip M, De Boer C, Pilpel D, Karplus M, Sofer S. Clitoral and penile sizes of full term newborns in two different ethnic groups. J Pediatr Endocrinol Metab. 1996 Mar-Apr;9(2):175-9.
  • 12. Kutlu HA, Akbiyik F. Clitoral length in female newborns: A new approach to the assessment of clitoromegaly. Turk J Med Sci. 2011;41:495-9.
  • 13. Dickinson RL.Atlas of Human Sex Anatomy. 2nd Ed. Baltimore: Williams & Wilkins. 1949; p. 1–145.
  • 14. Lloyd J, Crouch NS, Minto CL, Liao LM, Creighton SM. Female genital appearance: ‘Normality’ unfolds. BJOG. 2005;112:643–6.
  • 15. Oberfi eld SE, Mondok A, Shahrivar F, Klein JF, Levine LS. Clitoral size in full-term infants. American Journal of Perinatology. 1989;6:453-5.
  • 16. Verkauf BS,Von Thron J,O'Brien WF.Clitoral size in normal women. Obstet Gynecol. 1992;80:41–4.
  • 17. Poppas DP, Hochsztein AA, Baergen RN, Loyd E, Chen J, Felsen D. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol. 2007 Oct;178(4 Pt 2):1802-6; discussion 1806.
  • 18. Creighton SM. Surgery for intersex. J R Soc Med.2001;94:218-20.
  • 19. Crouch NS, Liao LM, Woodhouse CR, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol. 2008;179:634.
  • 20. Crouch NS, Minto CL, Laio LM, Woodhouse CR, Creighton SM. Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study. BJU Int. 2004;93:135.
  • 21. Baskin LS, Erol A, Li YW, Liu WH, Kurzrock E, Cunha GR. Anatomical studies of the human clitoris. J Urol. 1999 Sep;162(3 Pt 2):1015-20.
There are 21 citations in total.

Details

Primary Language English
Subjects Obstetrics and Gynaecology
Journal Section Research article
Authors

Adeviye Elçi Atılgan 0000-0003-4833-8978

Fatma Kılıç 0000-0002-9735-3552

Ali Acar 0000-0001-6478-2206

Tuğba Akçaoğlu 0000-0002-0354-3292

Asiye Uzun 0000-0001-8322-6643

Publication Date January 1, 2021
Published in Issue Year 2021 Volume: 5 Issue: 1

Cite

APA Elçi Atılgan, A., Kılıç, F., Acar, A., Akçaoğlu, T., et al. (2021). Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia. Journal of Surgery and Medicine, 5(1), 89-92. https://doi.org/10.28982/josam.865468
AMA Elçi Atılgan A, Kılıç F, Acar A, Akçaoğlu T, Uzun A. Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia. J Surg Med. January 2021;5(1):89-92. doi:10.28982/josam.865468
Chicago Elçi Atılgan, Adeviye, Fatma Kılıç, Ali Acar, Tuğba Akçaoğlu, and Asiye Uzun. “Nerve Sparing Feminizing Genitoplasty With Corporal Septum Excision in Non-Classic Congenital Adrenal Hyperplasia”. Journal of Surgery and Medicine 5, no. 1 (January 2021): 89-92. https://doi.org/10.28982/josam.865468.
EndNote Elçi Atılgan A, Kılıç F, Acar A, Akçaoğlu T, Uzun A (January 1, 2021) Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia. Journal of Surgery and Medicine 5 1 89–92.
IEEE A. Elçi Atılgan, F. Kılıç, A. Acar, T. Akçaoğlu, and A. Uzun, “Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia”, J Surg Med, vol. 5, no. 1, pp. 89–92, 2021, doi: 10.28982/josam.865468.
ISNAD Elçi Atılgan, Adeviye et al. “Nerve Sparing Feminizing Genitoplasty With Corporal Septum Excision in Non-Classic Congenital Adrenal Hyperplasia”. Journal of Surgery and Medicine 5/1 (January 2021), 89-92. https://doi.org/10.28982/josam.865468.
JAMA Elçi Atılgan A, Kılıç F, Acar A, Akçaoğlu T, Uzun A. Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia. J Surg Med. 2021;5:89–92.
MLA Elçi Atılgan, Adeviye et al. “Nerve Sparing Feminizing Genitoplasty With Corporal Septum Excision in Non-Classic Congenital Adrenal Hyperplasia”. Journal of Surgery and Medicine, vol. 5, no. 1, 2021, pp. 89-92, doi:10.28982/josam.865468.
Vancouver Elçi Atılgan A, Kılıç F, Acar A, Akçaoğlu T, Uzun A. Nerve sparing feminizing genitoplasty with corporal septum excision in non-classic congenital adrenal hyperplasia. J Surg Med. 2021;5(1):89-92.